Rock Gail, Anderson David, Clark William, Leblond Pierre, Palmer Douglas, Sternbach Marion, Sutton David, Wells George
Division of Hematology and Transfusion Medicine, Ottawa Civic Hospital, University of Ottawa, Ottawa, ON, Canada.
Br J Haematol. 2005 Apr;129(1):79-86. doi: 10.1111/j.1365-2141.2005.05418.x.
A randomized prospective trial compared cryosupernatant plasma (CSP) to fresh frozen plasma (FFP) for treatment of thrombotic thrombocytopenic purpura (TTP). A total of 236 patients were required: 28 patients were treated with CSP and 24 with FFP within 30 months. There were no differences in survival at 1 month. By day 9, 17 of 26 patients with CSP and 18 of 24 with FFP had a platelet count >100 x 10(9)/l. At entry, von Willebrand factor (VWF) multimers were normal in all patients (range 1.1-3.95 IU/ml). ADAMTS-13 levels showed large variations ranging from 10% to 100% activity. At entry, no individual had <5% VWF cleaving protease. By day 9 (end of cycle), 89% (FFP) and 67% (CSP) had levels >50% of the controls. At 6 months some patients showed inhibitors to the enzyme in spite of adequate or normal platelet counts. The data from this study do not show an apparent advantage to the use of CSP in TTP. A large number of patients will be required to determine appropriate replacement therapy. We were not able to find a statistically significant relationship between the low level of protease activity at presentation of TTP and response.
一项随机前瞻性试验比较了冷上清血浆（CSP）和新鲜冰冻血浆（FFP）治疗血栓性血小板减少性紫癜（TTP）的效果。共需要236例患者：在30个月内，28例患者接受CSP治疗，24例接受FFP治疗。1个月时生存率无差异。到第9天，26例接受CSP治疗的患者中有17例血小板计数>100×10⁹/L，24例接受FFP治疗的患者中有18例血小板计数>100×10⁹/L。入组时，所有患者的血管性血友病因子（VWF）多聚体均正常（范围为1.1 - 3.95 IU/ml）。ADAMTS - 13水平显示出较大差异，活性范围为10%至100%。入组时，没有个体的VWF裂解蛋白酶<5%。到第9天（周期结束时），89%（FFP）和67%（CSP）的水平>对照组的50%。6个月时，尽管血小板计数充足或正常，但一些患者显示出对该酶的抑制剂。这项研究的数据并未显示在TTP中使用CSP有明显优势。需要大量患者来确定合适的替代疗法。我们未能在TTP发病时蛋白酶活性水平低与反应之间找到统计学上的显著关系。
